Indonesian Journal of Cardiology Indonesian J Cardiol 2017:38:218-25 pISSN: 0126-3773 / eISSN: 2620-4762 Case Report

Pulmonary Atresia with Intact Ventricular Septum in a Neonate

Emir Yonas1, Raymond Pranata2, Nuvi Nusarintowati3 ,

Introduction: with an intact ventricular septum is a condition that is characterized by a complete obstruction to right ventricular outflow with varying degrees of right ventricular and tricuspid valve hypoplasia. This condition is uniformly fatal if untreated. In this case report, we present a case of a neonate with a pulmonary atresia with intact ventricular septum Case Presentation: A 2 days-old female Indonesian newborn was referred to our facility. The newborn was delivered from a G4P3A0 mother with a gestational age of 39 weeks (term delivery). Chest x-ray done at the referring facility is significant for a seemingly right hypertrophy, casting a “boot-shaped” appearance of the right heart border. Laboratory results done at the referring facility is significant for a neutrophilia of 82% and lymphocytopenia of 13% An episode of hypoglycemia was reported on referring facility with a sugar level of 50 mg/dl. Multiple episodes of were reported at referring facility, CPAP was administered but the improvement was limited. Pulse oximetry averaged between 72-80% at the referring facility. Physical examina- tion shows an actively moving neonate, with a strong cry. Perioral cyanosis was seen. Vital signs were as follows; heart rate 128 x / minute, temperature 37oC, respiratory 1Faculty of Medicine, rate 54 x / minute, pulse oximetry 74%, capillary refill time < 3 seconds. The rest of YARSI University, the physical examination was within normal limits. Laboratory result on admission at Jakarta, Indonesia our facility is significant for low hematocrit, low erythrocyte count, leukopenia with 2Faculty of Medicine, neutrophilia and thrombocytopenia. Patient was admitted to NICU and given CPAP. Universitas Pelita 2 days upon arrival at our facility reveals a patent Harapan, Tangerang, with the pressure of 4-5 mmHg and diameter of 0.3 cm, no forward flow from the right Indonesia ventricle to the , doppler mode demonstrated pulmonary artery filling 3Pediatric Cardiology from ductus arteriosus. The rest of the findings were within normal limits. division, University Conclusion: Pulmonary atresia with an intact ventricular septum is a condition that Of Indonesia, Jakarta, relies on the patent ductus arteriosus for pulmonary blood supply, the patency of it is Indonesia of paramount importance. (Indonesian J Cardiol. 2017;38:218-25)

Keywords: Pulmonary Atresia, Patent Ductus Arteriosus, congenital heart disease, hypoxic spells, cyanosis

218 Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017

Indonesian Journal of Cardiology Indonesian J Cardiol 2017:38:218-25 Laporan Kasus pISSN: 0126-3773 / eISSN: 2620-4762

Atresia Pulmonal dengan Septum Ventrikel Intak pada Neonatus

Emir Yonas1, Raymond Pranata2, Nuvi Nusarintowati3 ,

PENDAHULUAN: Atresia arteri pulmonalis dengan septum ventrikel yang utuh merupakan kondisi yang dikarakterisasikan dengan sumbatan total pada right ventricular outflow dengan berat hipoplasia ventrikel kanan dan trikuspid kanan yang beratnya bervariasi. Pada umumnya kondisi tersebut fatal apabila tidak ditangani. Pada laporan kasus ini, kami melaporkan suatu kasus neonates dengan atresia arteri pulmonalis dengan septum ventrikel yang utuh. PRESENTASI KASUS: Neonatus perempuan berumur 2 hari dirujuk ke rumah sakit kami. Bayi dilahirkan dari G4P3A0 usia kehamilan 39 minggu. Ronsen thoraks yang dilakukan oleh fasilitas kesehatan yang merujuk memperlihatkan hipertrofi jantung kanan serta bentuk “boot- shaped” pada batas jantung kanan. Hasil laboratorium pada fasilitas kesehatan tersebut menunjukan neutrofilia dan limfositopenia. Terdapat juga riwayat hipoglikemi. Episode sianosis berulang terjadi pada fasilitas kesehatan yang merujuk. Pemasangan CPAP kurang membuahkan hasil dengan saturasi O2 72-80% ketika tiba pada rumah sakit kami. Pemeriksaan fisik menunjukan tangis kuat dan gerak aktif dengan sianosis perioral. Detak jantung 128 kali/menit, suhu 37oC, laju naas 54 kali/menit, oksimetri nadi 74% dan waktu isi kapiler <3 detik. Pemeriksaan laboratorium saat pasien dating menunjukan hematocrit, hitung eritrosit, leukosit dan thrombosit yang rendah. Pasien dirawat di NICU dan diberikan CPAP. Ekokardiografi 2 hari kemudian menunjukan duktus arteriosus patent dengan tekanan 4-5 mmHg dan diameter 0.3 cm, tidak ada aliran maju dari ventrikel kanan kepada arteri pulmonalis, mode Doppler menunjukan pengisian arteri pulmonalis dari duktus arteriosus. Pemeriksaan lainnya dalam batas normal. KESIMPULAN: Atresia arteri pulmonalis dengan septum ventrikel yang utuh merupakan suatu kondisi dimana aliran darah menuju paru bergantung pada duktus arteriosus yang paten sehingga patensi daripada duktus tersebut sangatlah penting.

(Indonesian J Cardiol. 2017;38:218-25)

Kata Kunci: Atresia pulmonalis, duktus arteriosus paten, penyakit jantung bawaan, periode hipoksik, sianosis

Introduction

Pulmonary atresia with an intact ventricular septum is a condition that is characterized by a complete obstruc- 1Fakultas Kedokteran, Universitas YARSI, Jakarta, Indonesia, tion to right ventricular outflow with varying degrees 2Fakultas Kedokteran, Universitas Pelita Harapan, Tangerang, of right ventricular and tricuspid valve hypoplasia. As 3 Indonesia, Divisi Kardiologi Pediatrik, Universitas Indonesia, a result of these defects, blood is thus unable to flow Jakarta, Indonesia from the right ventricle to the pulmonary artery and Correspondence: lungs, and an alternative source of blood flow is then Dr.Emir Yonas, Facult of Medicine, YARSI University, Jakarta, required for survival. This condition is uniformly fatal Indonesia. E-mail: [email protected] if untreated.

Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 219 Indonesian Journal Of Cardiology

In this case report, we present a case of a neonate with cytopenia of 13% (normal range for individuals < 1 a pulmonary atresia with intact ventricular septum years old: 20-70%), the rest of laboratory values are within normal limits. An episode of hypoglycemia was reported on Case Presentation referring facility with a blood sugar level of 50 mg/ dl, subsequently, the neonate was given dextrose 10% A female Indonesian newborn was referred to our facility. with normal saline. No further episodes are reported. Due to suspicion of cyanotic heart disease. The newborn Multiple episodes of cyanosis were reported at referring was delivered 2 days prior to referral to our facility via facility, CPAP was administered but the improvement cesarean section. The newborn was delivered from a was limited. Pulse oximetry averaged between 72-80% G4P3A0 mother with a gestational age of 39 weeks (term at the referring facility. delivery). Weight on delivery was 2500 grams, body Upon arrival at our institution, a physical examina- length 47 centimeters, with an Apgar score at 1 and 5 tion shows an actively moving neonate, with a strong minutes of 7 and 9 respectively. She was referred to our cry. Perioral cyanosis was seen. Vital signs were as fol- facility aged 48 hours. An inquiry of the gestational his- lows; heart rate 128 x / minute, temperature 37oC, tory reveals a term, appropriate for gestational age . respiratory rate 54 x / minute, pulse oximetry 74%, Maternal age at current pregnancy was 38 years old. The capillary refill time < 3 seconds. Cardiac auscultation mother had 1 vaginal delivery and 2 cesarean section pre- reveals single and decreased second heart sound, with viously. The mother denied any history of sickness during no other murmur audible. The rest of the physical pregnancy. Patient’s parents denied any history of illnesses examination was within normal limits. of the heart and lungs in the family. Chest x-ray done at Laboratory result on admission at our facility is the referring facility is significant for a seemingly right significant for low hematocrit of 37% (normal range heart hypertrophy, casting a “boot-shaped” appearance 42-60%), low erythrocyte count of 3.8 mil/ microliter of the right heart border. Assessment of lung vasculature (normal range 3.9-5.5 mil/microliter), leukopenia with on CXR reveals an oligemic lung. WBC count of 3190 (normal range 9000-30,000 / The neonate was given intramuscular vitamin K, microliter), thrombocytopenia with count of 142000 ophthalmic antibiotic drops, normal saline intrave- / microliter (normal range 150,000-400,000 / micro- nously and cefotaxime (2x 125 mg, 4 doses in total) liter). Differential counting of white blood cell showed at referring facility. neutrophilia of 73% (normal range 50-70%). Laboratory results done at the referring facility is A suspicion of PAIVS was established by physical significant for a neutrophilia of 82 % (normal range examination and chest X ray, the neonate was then for individuals < 1 years old: 17-60%) and lympho- admitted to our neonatal intensive care unit. Continu-

Figure 1. Patient’s echocardiography showing patent ductus Figure 2. Patient’s echocardiography showing patent ductus arteriosus filling from the arteriosus width

220 Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 Yonas E et al: Pulmonary Atresia with Intact Ventricular Septum in a Neonate ous positive airway pressure was administered with a PAIVS is an acquired lesion due to perturbation in fraction of inspired oxygen of 30% and pressure of 5 fetal blood flow, based on reports of serial ultrasound cmH2O. studies showing the development of pulmonary atre- Echocardiography 2 days upon arrival at our facil- sia in . This theory has led to fetal interventions ity reveals a patent ductus arteriosus with the pressure including in utero dilation of the pulmonary valve of 4-5 mmHg and diameter of 0.3 cm, no forward to promote blood flow through the right ventricular flow from the right ventricle to the pulmonary artery, outflow tract.3,4,5 doppler mode demonstrated pulmonary artery filling In some other cases, it has been proposed that from ductus arteriosus. Monopartite right ventricular viral infection or inflammatory conditions in midterm morphology was seen. Pulmonal valve morphology gestation creates an atretic pulmonary valve and the was unable to be visualized. The rest of the findings subsequent abnormalities of fetal blood flow which were within normal limits. As per consultation with result in the occurrence of PAIVS. However, as per the pediatric cardiologist, it is advised the patient be current observations, no relationship has been estab- administered E1 infusion at the rate of lished between PAIVS and maternal , despite 10-30 nanogram/KgBW/minute intravenously in the the reported associations.6,7 event of desaturation. While on the other hand, pulmonary atresia with a ventricular septal defect, is thought to be an early gestational developmental lesion resulting from an Discussion abnormality in primary morphogenesis of the heart. PAIVS consists of a wide variety of anatomical Pulmonary Atresia with intact ventricular septum cardiac defects that are associated with the primary (PAIVS) is a rare congenital defect that consists of lesion of pulmonary atresia. atresia of the pulmonary valve resulting in an absent In patients with PAIVS, the underlying abnormality connection between the right ventricular outflow tract of the pulmonary atresia is either valvular or muscular and the pulmonary arteries, and an intact ventricular in origin. Most cases are caused by an atretic pulmonary septum that allows no connection between the right valve with a small valve annulus and identifiable but and left ventricles. fused valve leaflets which form a thin intact membrane It is to be considered that PAIVS is a distinct defect that causes right ventricular outflow tract obstruction. compared to pulmonary atresia in ventricular septal In these patients, the RV and infundibulum shaped defect and Ebstein anomaly. It is different that PAIVS muscular structure that forms the right ventricular features developmental abnormalities of the right ven- outflow tract below the pulmonary valve are usually tricle with the tricuspid valve that is upstream of the well developed.8,9 pulmonary outflow. The pulmonary arteries in PAIVS In approximately 20 to 25 percent of patients, are small, but the architecture and branching are nor- there is an obliteration of the muscular infundibulum mal. While in pulmonary atresia in ventricular septal resulting in muscular pulmonary atresia. This finding defect, the right ventricle is normally-formed and the is usually associated with severe right ventricular hyp- relation between the tricuspid valve and the pulmonary oplasia, increased risk, and severity of coronary artery artery is inverted in that downstream abnormalities abnormalities and poor outcome.8,10,11 of the pulmonary arteries are present, compared with A right ventricular defect is associated with the upstream abnormalities of PAIVS pulmonic valve abnormality. A great variation of the The reported incidence of this condition is based morphology of the right ventricle can be observed. It on registries and population-based studies of children ranges from a severely hypoplastic small cavity with born with congenital heart disease are approximately marked muscular hypertrophy to a large, dilated, thin- four to eight per 100,000 live births. PAIVS itself walled chamber. This variation is due to the degree of accounts for 1 to 3 percent of all congenital cardiac intracavitary muscular overgrowth. Normally the right defects in children regardless of gender.1,2 ventricle is composed of three parts; an inlet, a body The pathogenesis of this condition is unknown. and an outlet. Compared to other cardiac lesions, identifiable ge- Majority of patients will have a well formed tripar- netic syndromes and other cardiac malformations are tite right ventricle (59 to 83 percent), while the rest less common in PAIVS. It has been proposed that will have a bipartite right ventricle which is caused by

Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 221 Indonesian Journal Of Cardiology the overgrowth of apical trabecular tissues in a small pressure of the right ventricle can exceed systemic levels, ventricle which consist of an inlet, a body, but no outlet reaching as high as 200 mmHg in some cases. While (15 to 34 percent), small number of patient will have on the other hand, the right ventricular pressure can be unipartite right ventricle due to overgrowth of both normal in patients with tricuspid regurgitation. infundibular and apical trabecular tissues, resulting in The patency of ductus arteriosus is of paramount hypoplastic right ventricle with only a detectable inlet importance in PAIVS, it is crucial that the ductus (2 to 8 percent).8,10 kept open even after birth, as it is the only source of The size of the tricuspid valve is highly variable pulmonary blood supply. and the valve is often and dysplastic with stenosis and Cyanosis is present in neonates with PAIVS due or regurgitation. However, it is to be noted that the to an obligate right to left shunt at the atrial level. Se- size of the tricuspid annulus correlates with the size vere respiratory distress in an uncommon finding and and morphology of the right ventricle and it will be the occurrence differentiate these patients from those used as a consideration in deciding. the choice of in- with the pulmonary cause of cyanosis. However, mild tervention, in the terms of biventricular repair versus tachypnea and hyperpnea are common findings.10 an univentricular palliation.12 An untreated PAIVS is an almost uniformly fatal Patients with PAIVS will have abnormalities in disease. Pulmonary atresia with an intact ventricular the coronary arteries. Abnormal connections between septum is a ductal dependent lesion, closure of the pat- the right ventricle and coronary arteries are commonly ent ductus arteriosus generally results in rapid clinical found, this is thought to be caused by the high pres- deterioration and life-threatening consequences, which sure in the dysplastic right ventricular cavity. Patients includes severe metabolic acidosis, , seizures, with a severely hypoplastic tricuspid valve and a small cardiogenic shock, cardiac arrest, and death.18 hypertensive right ventricle are more likely to have In addition to cyanosis, other physical findings may fistulae. Coronary stenoses and interruptions will occur include a single second heart sound due to a single due to fistula formations.13,14,15 semilunar valve, systolic murmur due to tricuspid Pulmonary circulation in PAIVS is usually sup- regurgitation, and albeit less commonly, a continu- ported by normal left-sided patent ductus arteriosus, ous machinery type murmur due to the patent ductus as what is found in this patient, which connects to arteriosus. Pulse oximetry will reveal desaturation that confluent pulmonary arteries with a normal branching consistent with physical findings of cyanosis. It is to pattern from the main pulmonary trunk. It is rare for be noted that the systemic oxygen saturation depends patients with PA IVS to have abnormal development on the amount of left to right shunt from the aorta to of pulmonary arteries.16 the pulmonary arteries via the PDA. Although gross anatomic abnormalities of the Echocardiography is essential in confirming the di- pulmonary vasculature in PAIVS is uncommon, one agnosis of PAIVS, it also provides information regarding histologic study shows a significant medial and intimal the size and function of the right ventricle. Echocar- abnormalities, possibly related to the variation of pres- diography is also capable to evaluate the intracardiac sure due to ductal dependent pulmonary blood flow mixing of blood and the pulmonary blood flow. The compared to normal individuals.17 evaluation that can be done using echocardiography Other less common defects can occur in PAIVS, includes the following; (1) assessment of size and func- it usually affects the left ventricle, aortic valve, and tion of right ventricle, (2) measurement of tricuspid right atrium and thus are not pathognomonic for this valve size and function, (3) assessment of intracardiac condition mixing and pulmonary blood flow, (4) assessment of left In PAIVS, pulmonary atresia causes right ventricu- ventricular function, aortic valve, and left ventricular lar outflow obstruction. Due to the normal ventricular outflow tract. septum, there are only two ways left for blood to exit the Further evaluation regarding the patency and pos- right ventricle: (1) via tricuspid regurgitation back into sible abnormalities of the coronary arteries can be done the right atrium and (2) through the connections with the through a cardiac catheterization. coronary artery circulation with the right ventricle. Initial stabilization of PAIVS emphasizes on ensur- The pressure of right ventricle is dependent on the ing adequate systemic oxygenation, while also stabilizing extent of right ventricular outflow. In cases with limited the cardiac and pulmonary function. The goal of the tricuspid regurgitation and coronary circulation flow, the initial approach is to stabilize and optimize the neonate’s

222 Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 Yonas E et al: Pulmonary Atresia with Intact Ventricular Septum in a Neonate condition so that further repairs can be attempted. tricuspid valve is possible Pharmacological therapy is directed towards providing • Cardiac catheterization is performed on almost all sufficient intracardiac mixing of blood by ensuring the patients with PAIVS, the purpose of this is to assess patency of the ductus arteriosus with a prostaglandin the existence of right ventricle-dependent coronary E1 infusion. It is to be noted that the circulation (RVDCC), which will impose as a in this condition is dependent on blood shunting from barrier to proceed to biventricular repair, patients the right to the left atrium. General cardiorespiratory with RVDCC will typically undergo palliative ap- support for infants that suffers from either respiratory proaches. compromise, poor perfusion, acidosis, and or hypoten- sion will also be included in the initial management of Furthermore, in the setting of marked cyanosis PAIVS. Measures to counteract these derangements will not responding to pharmacologic agents, patients can include respiratory support with either supplemental undergo balloon valvuloplasty of the pulmonary valves, oxygen or mechanical ventilation, inotropic agents, and patients with pulmonary atresia are candidates for bal- correction of metabolic derangements.8 loon valvuloplasty, provided that the morphology of There are several options for corrective surgical obstruction is membranous, the right ventricular size is repair or palliation for patients with PAIVS, the choice adequate for repair, and there’s no RVDCC. However, of approach will best be determined by pediatric cardi- as our patient exhibited improvement after administra- ologists and cardiothoracic surgeons. These approaches tion of prostaglandin, this is not necessary. can include;19,20 This patient experienced multiple episodes of desat- • A biventricular repair, in which separation of urations, in which continuous positive airway pressure pulmonary and systemic circulations with two ventilation was implemented, albeit the limited im- pumping ventricles will be done provement, this is caused by a right to left shunting that • Univentricular palliation, which will separate the occurs as the right ventricular outflow tract is obstructed. pulmonary and systemic circulations with a single No amount of oxygenation can correct the subnormal pumping ventricle levels of oxygen in this patient’s blood due to it being • Hybrid/1.5 ventricle repair, which separates the caused by the intracardiac mixing of oxygenated and pulmonary and systemic circulations with two deoxygenated blood. If one were to do blood gas analysis pumping ventricles, with the exception that the on this patient and count the alveolar-arterial oxygen right ventricle is not totally supporting the pul- gradient of this patient, it will show results exceeding 15 monary circulation by itself. In this approach, point due to it being caused by shunting. Prostaglandin Glenn procedure will be performed which will E1 was administered in the setting of desaturation, and then provides a direct source of pulmonary blood the patient improved shortly afterward. flow from the , while the infe- From echocardiography result of this patient, it rior vena cava is channeled to the right ventricle is apparent that the blood flow to the pulmonary and the reconstructed pulmonary valve. At a later vasculature originated from the aorta via the ductus time when right ventricular growth is sufficient to arteriosus, as demonstrated by the Doppler mode support the pulmonary circulation, catheter-based echocardiography result of this patient. Echocardiog- closure of the patent foramen ovale or atrial septal raphy also shows an atrial septal defect, which acts as defect can be attempted. a conduit of blood from the right side of the heart to • Primary cardiac transplantation the left side of the heart. The implementation of ventilation support in The management of PAIVS is a combined medical cases of PAIVS is not without its risks, it is a well- and surgical effort, with the burden of decision mak- established fact that at birth, the rise in systemic ing shared on both disciplines and on anatomical and arterial oxygen tension, and a decrease in circulating physiological data of the lesion, to assess the feasibility levels of are the main factors that of biventricular repair. Several methods are available to induce the constriction and subsequent obliteration help provide morphologic data that will help decision of ductus arteriosus. The administration of supple- making in the choice of management: mental oxygen will further hasten the closure of • Echocardiography is done to measure the size and ductus arteriosus in these cases, which will bring morphology of the right ventricle and to assess the catastrophic consequences and rapid clinical dete-

Indonesian J Cardiol • Vol. 38, Issue 4 • October - December 2017 223 Indonesian Journal Of Cardiology rioration as the pulmonary blood flow is obliterated. Consent for publication In the face of these considerations, it is dangerous to continue oxygen supplementation without estab- Written informed consent was obtained from the lishing the echocardiographic diagnosis in cases of patient’s legal guardian(s) for publication of this case suspected PAIVS. report and any accompanying images. A copy of the As the ductus arteriosus remains a critical anatomic written consent is available for review by the Editor- part prior to corrective , it is highly important in-Chief of this journal that it kept open, in this case, we advised the admin- istration of infusion at the rate of 10-30 nanogram/KgBW/minute intravenously in the Availability of data and material event of desaturation, in order to maintain patency of ductus arteriosus. The datasets supporting the conclusions of this article The authors acknowledged that there are limita- are included in the article tions to this case report, such as the use of CPAP in this patient, which has limited benefit and has been ceased Competing interests after consultation with the pediatric cardiologist. We are also unable to determine the existence of RVDCC and The authors declare that they have no competing z score calculation at the time of this case report due to interests the patient not undergoing catheterization at our facil- ity and being referred to another tertiary care center to Funding undergo further management. No source of funding Conclusion Authors’ contributions

Pulmonary atresia with an intact ventricular septum is NN admitted, evaluate and treat the patient. EY and a condition that relies on the patent ductus arteriosus RP also planned and drafted the manuscript. EY for pulmonary blood supply, the patency of it is of and RP performed extensive research on the topic. paramount importance. Therefore, it is dangerous to NN supervised and gave expert advice regarding the continue oxygen supplementation without a proper manuscript. All authors read and approved the final diagnosis as it can cause ductal closure and rapid de- manuscript terioration of the patient. Acknowledgements

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